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ALAS1 Recombinant Monoclonal Antibody

  • 中文名稱:
    ALAS1 Recombinant Monoclonal Antibody
  • 貨號:
    CSB-RA220019A0HU
  • 規格:
    ¥1320
  • 圖片:
    • Flow cytometric analysis of ALAS1 expression in HAP-1 cells using ALAS1 antibody. Green, isotype control; red, ALAS1.
    • Immunocytochemical staining of HAP-1 cells with ALAS1 antibody. Nuclei were stained blue with DAPI; ALAS1 was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: Medium. Scale bar, 20 μm.
    • Western blotting analysis using ALAS1 antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with ALAS1 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    ALAS1
  • 別名:
    ALAS1; 5'-Aminolevulinate Synthase 1; ALAS-H; ALAS3; 5-Aminolevulinate Synthase, Non-Specific, Mitochondrial; Aminolevulinate, Delta-, Synthase 1; 5-Aminolevulinic Acid Synthase 1; Delta-Aminolevulinate Synthase 1; Delta-ALA Synthase 1; EC 2.3.1.37; ALASH; ALAS; 5-Aminolevulinate Synthase, Nonspecific, Mitochondrial; Migration-Inducing Protein 4; EC 2.3.1; MIG4
  • 反應種屬:
    Human, Mouse, Rat
  • 免疫原:
    Recombinant Human ALAS1 protein
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 克隆類型:
    Monoclonal
  • 抗體亞型:
    Rabbit IgG
  • 純化方式:
    Affinity-chromatography
  • 克隆號:
    14D11
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Rabbit IgG in PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, WB, FC, ICC
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:1000-1:5000
    FC 1:200-1:2000
    ICC 1:100-1:1000
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 基因功能參考文獻:
    1. The mutation in CLPX inactivates its ATPase activity, resulting in coassembly of mutant and WT protomers to form an enzyme with reduced activity. The presence of low-activity CLPX increases the posttranslational stability of ALAS, causing increased ALAS protein and ALA levels, leading to abnormal accumulation of PPIX. PMID: 28874591
    2. the heme-binding site in the N-terminal region of the mature ALAS1 protein is also necessary for the heme-dependent oxidation of ALAS1. PMID: 27496948
    3. ALAS1 mRNA and activity were elevated approximately ~3- and 5-fold, and HMB synthase activity was approximately half-normal (~42%) PMID: 26062020
    4. The -853T variant functions as an enhancer in the presence of estrogen and speculates that the -1253A variant reduces transcription activity. PMID: 19656447
    5. These results indicate that ALAS1 is a novel NR5A-target gene and participates in steroid hormone production. PMID: 23024262
    6. Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells. PMID: 21659532
    7. REVIEW:active site and mechanistic analysis, protein folding, structure and function. PMID: 11929042
    8. REVIEW: mechanisms involving ALAS deficiency, point mutations, post translational processing, and complex formation with succinyl CoA synthetase subunit B in the pathogenesis of hereditary sideroblastic anemia. PMID: 11929048
    9. ALAS expression is regulated by AP-1 complex through sequestration of cAMP-response element protein (CRE)-binding protein (CBP) coactivator in human cells PMID: 12433930
    10. ALAS gene expression is regulated by Hepatic nuclear factor 3 and nuclear factor 1 PMID: 15123725
    11. First described frameshift ALAS2 mutation, CD506-507 (-C). PMID: 15477213
    12. in the liver of Acute liver failure patients, there may be an increase in free heme concentration which down-regulating ALAS1 gene expression PMID: 15547665
    13. Alternative splicing of human ALAS1 generates two mRNAs with different 5'-UTRs: a major one, where exon 1B is omitted, and a minor form containing exon 1B. PMID: 15710391
    14. 5-aminolevulinate synthase gene repression by the potent tumor promoter, TPA, involves multiple signal transduction pathways PMID: 15797241
    15. Expression of candidate genes HPRT1 and ALAS1 in malignant and non-malignant prostate tissue samples after microdissection. PMID: 17628775
    16. Differential regulation of human ALAS1 mRNA and protein levels by heme and cobalt protoporphyrin. PMID: 18719978
    17. In this study, we show significant reductions of the rate-limiting enzymes involved in heme biosynthesis, ALAS1 in the postmortem cortex of Alzheimer's disease subjects, providing additional evidence of abnormal heme homeostasis in Alzheimer's disease. PMID: 19477221

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  • 亞細胞定位:
    Mitochondrion matrix.
  • 蛋白家族:
    Class-II pyridoxal-phosphate-dependent aminotransferase family
  • 數據庫鏈接:

    HGNC: 396

    OMIM: 125290

    KEGG: hsa:211

    STRING: 9606.ENSP00000309259

    UniGene: Hs.476308



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